Henoch-Schonlein purpura: Difference between revisions

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===Classic===
===Classic===
[[File:HSPVasc01.jpg| thumb|Palpable Purpura]]
[[File:HSPVasc01.jpg| thumb|Palpable Purpura]]
''May develop over the course of days to weeks and may vary in their order of presentation''
''May develop over the course of days to weeks and may vary in order of presentation''
#Rash: palpable purpura
#Rash: palpable purpura
#*Often begins with erythematous, macular, or urticarial wheals, progressing into the typical ecchymoses, petechiae, and palpable purpura (see picture)
#*Often begins with erythematous, macular, or urticarial wheals, progressing into the typical ecchymoses, petechiae, and palpable purpura (see picture)

Revision as of 17:59, 27 October 2015

Background

  • Most common vasculitis in childhood
    • Small vessel
  • Most cases preceded by a URI
  • Usually affects 2-11 yr
  • 5% of cases associated with intussusception (abdominal vasculitis)
  • Renal involvement is feared complication
  • 95% recover completely after 3-4wk

Clinical Presentation

Classic

Palpable Purpura

May develop over the course of days to weeks and may vary in order of presentation

  1. Rash: palpable purpura
    • Often begins with erythematous, macular, or urticarial wheals, progressing into the typical ecchymoses, petechiae, and palpable purpura (see picture)
    • Typically appears in crops, symmetrically distributed, and located primarily in gravity/pressure-dependent areas (e.g. lower extremities, buttocks)
  2. Acute abdominal pain
    • Typically diffuse and colicky
    • May have blood in the stool (massive bleeding is rare)
  3. Arthritis/arthralgia
    • Migratory, usually involves knees/ankles
  4. Nephritis/renal disease
    • (~50% of the time)[1]
    • Hematuria +/- proteinuria

Rare manifestations

  • Melena, hematemesis, hepatosplenomegaly
  • Headache, seizures
  • Fever
  • Non-pitting edema of the extremities and face
  • Nephrotic Syndrome
    • Long-term mortality directly related to renal involvement[2][3]

Differential Diagnosis

Pediatric Rash

Causes of Glomerulonephritis

Diagnosis

Work-Up

  1. UA
    • Hematuria, proteinuria
  2. Chemistry
  3. Consider stool guaiac if concern for melena

Treatment

  • Supportive
  • NSAIDs for pain, may worsen renal disease or GI disease
  • Consider prednisone 1mg/kg/day for severe arthralgias, abdominal or scrotal disease

Disposition

  • Outpt management for most w/ rheum f/u
  • Recurrence rate of up to 33%

See Also

References

  1. Chen JY et al. Henoch-Schönlein purpura nephritis in children: incidence, pathogenesis and management. World J Pediatr. 2015 Feb;11(1):29-34. doi: 10.1007/s12519-014-0534-5. Epub 2014 Dec 29.
  2. Calviño, MC, Llorca, J, García-Porrúa, C, Fernández-Iglesias, JL, Rodriguez-Ledo, P, González-Gay, MA (2001) Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine (Baltimore) 80: pp. 279-290
  3. Saulsbury, FT (1999) Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 78: pp. 395-409